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Recent advances in understanding and treating immunoglobulin light chain amyloidosis
Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical co...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
F1000 Research Limited
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117860/ https://www.ncbi.nlm.nih.gov/pubmed/30228867 http://dx.doi.org/10.12688/f1000research.15353.1 |
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author | Badar, Talha D'Souza, Anita Hari, Parameswaran |
author_facet | Badar, Talha D'Souza, Anita Hari, Parameswaran |
author_sort | Badar, Talha |
collection | PubMed |
description | Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis. Treatment for patients with AL amyloidosis should be risk-adapted and customized on the basis of individual patient characteristics. In the future, approaches directed at amyloid fibril clearance in combination with agents that target plasma cells will be needed both to eradicate the malignant clone and to establish organ responses. |
format | Online Article Text |
id | pubmed-6117860 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | F1000 Research Limited |
record_format | MEDLINE/PubMed |
spelling | pubmed-61178602018-09-17 Recent advances in understanding and treating immunoglobulin light chain amyloidosis Badar, Talha D'Souza, Anita Hari, Parameswaran F1000Res Review Immunoglobulin (Ig) light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by misfolded Ig light chain deposition in vital organs of the body, resulting in proteotoxicity and organ dysfunction. Owing to its diverse clinical presentations and a tendency to mimic common medical conditions, AL amyloidosis is often diagnosed late and results in dismal outcomes. Early referral to a specialized center with expertise in management of AL amyloidosis is always recommended. The availability of sensitive biomarkers and novel therapies is reforming our approach to how we manage AL amyloidosis. Treatment for patients with AL amyloidosis should be risk-adapted and customized on the basis of individual patient characteristics. In the future, approaches directed at amyloid fibril clearance in combination with agents that target plasma cells will be needed both to eradicate the malignant clone and to establish organ responses. F1000 Research Limited 2018-08-29 /pmc/articles/PMC6117860/ /pubmed/30228867 http://dx.doi.org/10.12688/f1000research.15353.1 Text en Copyright: © 2018 Badar T et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Badar, Talha D'Souza, Anita Hari, Parameswaran Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title | Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title_full | Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title_fullStr | Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title_full_unstemmed | Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title_short | Recent advances in understanding and treating immunoglobulin light chain amyloidosis |
title_sort | recent advances in understanding and treating immunoglobulin light chain amyloidosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117860/ https://www.ncbi.nlm.nih.gov/pubmed/30228867 http://dx.doi.org/10.12688/f1000research.15353.1 |
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